Division of Colon and Rectal Surgery, Mayo Clinic, Rochester, MN, USA.
School of General Surgery, Alma Mater Studiorum University of Bologna, Bologna, Italy.
Ann Surg Oncol. 2024 Jul;31(7):4551-4557. doi: 10.1245/s10434-024-15328-3. Epub 2024 Apr 28.
Presacral neuroendocrine neoplasms (PNENs) are rare tumors, with limited data on management and outcomes.
A retrospective review of institutional medical records was conducted to identify all patients with PNENs between 2008 and 2022. Data collection included demographics, symptoms, imaging, surgical approaches, pathology, complications, and long-term outcomes.
Twelve patients were identified; two-thirds were female, averaging 44.8 years of age, and, for the most part, presenting with back pain, constipation, and abdominal discomfort. Preoperative imaging included computed tomography scans and magnetic resonance images, with somatostatin receptor imaging and biopsies being common. Half of the patients had metastatic disease on presentation. Surgical approach varied, with anterior, posterior, and combined techniques used, often involving muscle transection and coccygectomy. Short-term complications affected one-quarter of patients. Pathologically, PNENs were mainly well-differentiated grade 2 tumors with positive synaptophysin and chromogranin A. Associated anomalies were common, with tail-gut cysts prevalent. Mean tumor diameter was 6.3 cm. Four patients received long-term adjuvant therapy. Disease progression necessitated additional interventions, including surgery and various chemotherapy regimens. Skeletal, liver, thyroid, lung, and pancreatic metastases occurred during follow-up, with no mortality reported. Kaplan-Meier analysis showed a 5-year local recurrence rate of 23.8%, disease progression rate of 14.3%, and de novo metastases rate of 30%.
The study underscores the complex management of PNENs and emphasizes the need for multicenter research to better understand and manage these tumors. It provides valuable insights into surgical outcomes, recurrence rates, and overall survival, guiding future treatment strategies for PNEN patients.
直肠前神经内分泌肿瘤（PNENs）是一种罕见的肿瘤，其管理和预后数据有限。
对机构病历进行回顾性研究，以确定 2008 年至 2022 年间所有患有 PNENs 的患者。数据收集包括人口统计学、症状、影像学、手术方法、病理学、并发症和长期结果。
共确定了 12 名患者；三分之二为女性，平均年龄为 44.8 岁，大多数患者表现为背痛、便秘和腹部不适。术前影像学检查包括 CT 扫描和磁共振成像，生长抑素受体成像和活检很常见。一半的患者在就诊时已有转移疾病。手术方法多种多样，采用了前、后和联合技术，常涉及肌肉横断和尾骨切除术。近四分之一的患者出现短期并发症。在病理学上，PNENs 主要是分化良好的 2 级肿瘤，Synaptophysin 和 chromogranin A 阳性。常见伴发畸形，尾肠囊肿多见。肿瘤平均直径为 6.3cm。4 名患者接受了长期辅助治疗。疾病进展需要进一步干预，包括手术和各种化疗方案。在随访期间发生了骨骼、肝脏、甲状腺、肺和胰腺转移，无死亡报告。Kaplan-Meier 分析显示，5 年局部复发率为 23.8%，疾病进展率为 14.3%，新发转移率为 30%。
该研究强调了 PNENs 的复杂管理，并强调需要进行多中心研究以更好地理解和管理这些肿瘤。它提供了有关手术结果、复发率和总生存率的宝贵见解，为 PNEN 患者的未来治疗策略提供了指导。
